Article

Abnormal triaging of misfolded proteins by adult neuronal ceroid lipofuscinosis-associated CSPα mutants causes lipofuscin accumulation

2021-07-16

Abstract excerpt

Mutations in DNAJC5 (encoding the J domain-containing HSP70 co-chaperone CSPα) are associated with adult neuronal ceroid lipofuscinosis (ANCL), a dominant-inherited neurodegenerative disease featuring lysosome-derived a utofluorescent s torage m aterial (AFSM) termed lipofuscin. Functionally, CSPα has been implicated in chaperoning synaptic proteins and in misfolding-associated protein secretion (MAPS), but ho...

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Literature Corpus work: 9babfce8-cc81-5252-99c0-2cb82b43af5c
DOI: 10.1101/2021.07.16.452648

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